We report a case of a successful surgical resection of a Wilms’ tumor in the right kidney with a coincidental preoperative imaging finding of a left-sided inferior vena cava. To our knowledge, these 2 conditions occurring together has not been previously reported in literature. Diagnostic features and the value of magnetic resonance imaging are emphasized. We also review the literature of major venous anomalies and their influence on surgical procedures carried out on such patients. We report a case of a successful surgical resection of a Wilms’ tumor in the right kidney with a coincidental preoperative imaging of a left-sided inferior vena cava. To our knowledge, these 2 conditionsomenon together not not previously been reported in literature. Diagnostic features and the value of magnetic resonance imaging are emphasized. We also review the literature of major venous anomalies and their influence on surgical procedures carried out on such patients.